KEGG   ENZYME: 4.1.2.63
Entry
EC 4.1.2.63                 Enzyme                                 
Name
2-hydroxyacyl-CoA lyase;
HACL1 (gene name);
2-hydroxyphytanoyl-CoA lyase;
2-HPCL
Class
Lyases;
Carbon-carbon lyases;
Aldehyde-lyases
Sysname
2-hydroxy-3-methyl fatty-CoA formyl-CoA lyase (2-methyl branched fatty aldehyde-forming)
Reaction(IUBMB)
(1) a 2-hydroxy-3-methyl-Cn-fatty-acyl-CoA = a 2-methyl-branched Cn-1-fatty aldehyde + formyl-CoA [RN:R12799];
(2) a (2R)-2-hydroxy-Cn-long-chain fatty acyl-CoA = a Cn-1-long-chain fatty aldehyde + formyl-CoA [RN:R12800]
Reaction(KEGG)
R12799 R12800
Substrate
2-hydroxy-3-methyl-Cn-fatty-acyl-CoA;
(2R)-2-hydroxy-Cn-long-chain fatty acyl-CoA
Product
2-methyl-branched Cn-1-fatty aldehyde;
formyl-CoA [CPD:C00798];
Cn-1-long-chain fatty aldehyde
Comment
Requires Mg2+ and thiamine diphosphate. This peroxisomal enzyme, found in animals, is involved in the alpha-oxidation of 3-methyl-branched fatty acids like phytanic acid and the shortening of 2-hydroxy long-chain fatty acids.
History
EC 4.1.2.63 created 2021
Orthology
K12261  2-hydroxyacyl-CoA lyase
Genes
HSA26061(HACL1)
PTR743738(HACL1)
PPS100982158(HACL1)
GGO101130205(HACL1)
PON100457302(HACL1)
NLE100589385(HACL1)
MCC694261(HACL1)
MCF102130550(HACL1)
CSAB103241708(HACL1)
CATY105578325(HACL1)
PANU101016987(HACL1)
TGE112617609(HACL1)
RRO104656450(HACL1)
RBB108535627(HACL1)
TFN117084204(HACL1)
PTEH111539503(HACL1)
CJC100385052(HACL1)
SBQ101042137(HACL1)
CSYR103249270(HACL1)
MMUR105872704(HACL1)
OGA100942962(HACL1)
MMU56794(Hacl1)
MCAL110309041(Hacl1)
MPAH110325771(Hacl1)
RNO85255(Hacl1)
MCOC116084402(Hacl1)
MUN110551269(Hacl1)
CGE100767514(Hacl1)
PLEU114708165(Hacl1)
NGI103747520(Hacl1)
HGL101706829(Hacl1)
CPOC100731806(Hacl1)
CCAN109694810(Hacl1)
DORD105989902(Hacl1)
DSP122107948(Hacl1)
OCU100342558(HACL1)
OPI101524332(HACL1)
TUP102488706(HACL1)
CFA608697
VVP112925619(HACL1)
VLG121478711(HACL1)
AML100483076(HACL1)
UMR103679362(HACL1)
UAH113253912(HACL1)
UAR123788357(HACL1)
ELK111155096
LLV125104689
MPUF101679744(HACL1)
ORO101380715(HACL1)
EJU114205724(HACL1)
ZCA113915823(HACL1)
MLX118003367(HACL1)
FCA101093708(HACL1)
PYU121033647(HACL1)
PBG122492011(HACL1)
PTG102951290(HACL1)
PPAD109258811(HACL1)
AJU106971590(HACL1)
HHV120238469(HACL1)
BTA512676(HACL1)
BOM102283180(HACL1)
BIU109561790(HACL1)
BBUB102399404(HACL1)
CHX102182450(HACL1)
OAS101119009(HACL1)
ODA120880360(HACL1)
CCAD122445507(HACL1)
SSC100518218(HACL1)
CFR102508961(HACL1)
CBAI105077623(HACL1)
CDK105096613(HACL1)
VPC102525121(HACL1)
BACU103005299(HACL1)
LVE103081479(HACL1)
OOR101278567(HACL1)
DLE111168157(HACL1)
PCAD102980162(HACL1)
PSIU116753296(HACL1)
ECB100063920(HACL1)
EPZ103561485(HACL1)
EAI106842763(HACL1)
MYB102259094(HACL1)
MYD102760568(HACL1)
MMYO118668201(HACL1)
MLF102419042(HACL1)
MNA107542410(HACL1)
PKL118715364(HACL1)
HAI109395823(HACL1)
DRO112309688(HACL1)
SHON118997591(HACL1)
AJM119048308(HACL1)
PDIC114501580(HACL1)
PHAS123830654(HACL1)
MMF118633385(HACL1)
RFQ117037187(HACL1)
PALE102884140(HACL1)
PGIG120582055(HACL1)
PVP105288363(HACL1)
RAY107508452(HACL1)
MJV108390849(HACL1)
TOD119256849(HACL1)
SARA101543974(HACL1)
LAV100667352(HACL1)
TMU101354112
DNM101437479(HACL1)
MDO100030500(HACL1)
GAS123250584(HACL1)
SHR100916928(HACL1)
PCW110209056(HACL1)
OAA100084023(HACL1)
GGA420638(HACL1)
PCOC116227085(HACL1)
MGP100542074(HACL1)
CJO107309164(HACL1)
NMEL110394950(HACL1)
APLA101798319(HACL1)
ACYG106030248(HACL1)
AFUL116486217(HACL1)
TGU100232217(HACL1)
LSR110474620(HACL1)
SCAN103812914(HACL1)
PMOA120502665(HACL1)
OTC121339586(HACL1)
PRUF121352912(HACL1)
GFR102037543(HACL1)
FAB101821112(HACL1)
PHI102104383(HACL1)
PMAJ107199916(HACL1)
CCAE111923442(HACL1)
CCW104689075(HACL1)
ETL114055681(HACL1)
ZAB102070040(HACL1)
FPG101915302(HACL1)
FCH102057939(HACL1)
CLV102085572(HACL1)
EGZ104126084(HACL1)
NNI104013970(HACL1)
ACUN113476794(HACL1)
TALA104358157(HACL1)
PADL103918430(HACL1)
ACHC115339770(HACL1)
AAM106483474(HACL1)
AROW112977277(HACL1)
NPD112957212(HACL1)
DNE112985274(HACL1)
ASN102372627(HACL1)
AMJ102569780(HACL1)
CPOO109310399(HACL1)
GGN109291599(HACL1)
PSS102457478(HACL1)
CMY102946745(HACL1)
CPIC101939557(HACL1)
TST117872359(HACL1)
CABI116837022(HACL1)
MRV120398934(HACL1)
ACS100557174(hacl1)
PVT110072147(HACL1)
SUND121934086(HACL1)
PBI103048759(HACL1)
PMUR107282910(HACL1)
TSR106545060(HACL1)
PGUT117655106(HACL1)
VKO123033833(HACL1)
PMUA114607097(HACL1)
ZVI118092350(HACL1)
GJA107126170(HACL1)
XLA446657(hacl1.L)
XTR550086(hacl1)
NPR108802925(HACL1)
BBUF121002206(HACL1)
BGAR122938311(HACL1)
DRE406358(hacl1)
SRX107743162 107745383
SANH107684560 107685543
SGH107550801 107596724(hacl1)
CCAR109106433(hacl1)
CAUA113116276(hacl1)
IPU108267388(hacl1)
PHYP113542549(hacl1)
SMEO124385334(hacl1)
EEE113571243(hacl1)
TRU101061718(hacl1)
TNGGSTEN00034133G001
LCO104927751(hacl1)
NCC104944968(hacl1)
CGOB115021252(hacl1)
ELY117265388(hacl1)
PLEP121945760(hacl1)
SLUC116049391(hacl1)
ECRA117946488(hacl1)
PFLV114557200(hacl1)
GAT120811025(hacl1)
PPUG119198104(hacl1)
MSAM119897754(hacl1)
CUD121513857(hacl1)
MZE101465513(hacl1)
ONL100690060(hacl1)
OAU116325395(hacl1)
OLA101156193(hacl1)
OML112143126(hacl1)
XMA102223303(hacl1)
XCO114142081(hacl1)
XHE116717578(hacl1)
PRET103478561(hacl1)
PFOR103150389 103150392
PLAI106941218 106955235
PMEI106922205(hacl1)
GAF122831424
CVG107103875(hacl1)
CTUL119772782(hacl1)
GMU124865949(hacl1)
NFU107378691(hacl1)
KMR108241945(hacl1)
ALIM106517744(hacl1)
NWH119409454(hacl1)
AOCE111588844(hacl1)
CSEM103388412(hacl1)
POV109633118(hacl1)
SSEN122774464(hacl1)
HHIP117777286(hacl1)
LCF108891319(hacl1)
SDU111238787(hacl1)
SLAL111652800(hacl1)
XGL120784806(hacl1)
HCQ109525685(hacl1)
BPEC110171659(hacl1)
MALB109967232(hacl1)
SASA106592607(hacl1) 106596819
OTW112225223(hacl1)
OMY110505030(hacl1) 110515045 118936608
OGO124019490 124023920
SALP111971452(hacl1)
SNH120038022 120040290
ELS105018820(hacl1)
SFM108927371(hacl1)
PKI111839032(hacl1)
AANG118212187(hacl1)
LOC102687234(hacl1)
PSPA121315127(hacl1)
ARUT117394717(hacl1)
LCM102362798(HACL1)
CMK103181261(hacl1)
RTP109933345(hacl1)
BFO118407296
BBEL109468963
CIN100178631
SCLV120341709
SPU586415
APLC110973383
SKO100370616(HACL1)
DMEDmel_CG11208(Hacl)
DER6547754
DSE6610038
DSIDsimw501_GD25296(Dsim_GD25296)
DYADyak_GE13819
DAN6496000
DSR110183103
DPODpse_GA10842
DPE6600980
DMN108160101 117192789
DWI6640896
DGR6568724
DMODmoj_GI18529
DAZ108616686
DNV108654972
DHE111594678
DVI6625321
CCAT101448871
BOD106624777
MDE101901094
SCAC106096256
LCQ111684644
AGAAgaP_AGAP010368
ACOZ120956444
AARA120902070
AAG5565030 5572369
AALB109398509
CQUCpipJ_CPIJ014172 CpipJ_CPIJ017705
CPII120417246
CNS116340071
AME552738
ACER107995125
ALAB122717988
BIM100744016
BBIF117207092
BVK117237409
BVAN117160978
BTER100644973
BPYO122572768
CCAL108629887
OBB114878797
MGEN117229721
NMEA116434819
CGIG122404927
SOC105194976
MPHA105836739
AEC105154992
ACEP105626893
PBAR105426340
VEM105566829
HST105189078
DQU106750369
CFO105255983
FEX115243773
LHU105669841
PGC109857866
OBO105282994
PCF106784580
PFUC122516260
VPS122627482
NVI100123260
CSOL105361881
TPRE106658765
MDL103573582
CGLO123270220
FAS105272077
DAM107038575
AGIF122855352
CCIN107263840
TCA664029
DPA109536893
SOY115884215
ATD109596968
CSET123317367
AGB108903720
LDC111511260
NVL108560626
APLN108742675
PPYR116180506
OTU111426368
BMOR692884
BMAN114245011
MSEX115455821
DPLKGM_216038
BANY112045363
PMAC106707335 106712100
PPOT106110820
PXU106126854
PRAP110998130
ZCE119839860
HAW110380221
TNL113492403
OFU114354508
PXY105381789 105388089 105388092
API100165444
DNX107163705
AGS114132285
RMD113551822
BTAB109042181
DCI103515657
CLEC106674218
HHAL106686454
NLU111047026
PHUPhum_PHUM529400
FOC113211311
ZNE110831053
CSEC111868986
FCD110848423
DPXDAPPUDRAFT_253560 DAPPUDRAFT_306512
DMK116930937
PVM113809547 113829823
PJA122259379
HAME121861697
PCLA123761322
PTRU123499102
EAF111696878
DSV119442762
RSAN119387927 119387928
RMP119188213
VDE111248283
VJA111271184
CSCU111629264
PTEP107455176
SDM118180287 118202574
CELCELE_B0334.3(B0334.3)
CBRCBG_02874
NAINECAME_12882
LGILOTGIDRAFT_207618
PCAN112564745
BGT106078649
GAE121382197
HRF124141592 124141617
HRJ124281687
CRG105325181
MYI110449719
PMAX117343704
MMER123531889
OBI106872707
OSN115212767
LAK106162962 106178375
NVE5509052
EPA110232378 110241064 110251089
ATEN116307823
ADF107339514 107349360
AMIL114965786
PDAM113664752
SPIS111333468
DGT114536270
AQU100641768
ATHAT5G17380
ALY9309892
CRB17882398
CSAT104706028 104735774 104770038
EUSEUTSA_v10013104mg
BRP103833165
BNA106383786 106445967 106445973
BOE106318756
RSZ108817706
THJ104820853
CPAP110807471
CIT102621022
CICCICLE_v10011371mg CICLE_v10011616mg
PVY116111220
MINC123197953 123225748
TCC18601969
GRA105803616
GHI107912763 107912909
GAB108453368
DZI111305773 111314659
EGR104449677
GMX100778257 100781584
GSJ114384324 114397553
PVUPHAVU_008G243200g
VRA106765165
VAR108338133
VUN114195378
CCAJ109796119
APRC113847006
MTRMTR_6g004200
CAM101501749
LJALj2g3v0919440.1(Lj2g3v0919440.1)
ADU107489982
AIP107644561
AHF112751450 112801899
LANG109347251
FVE101306541
RCN112195257
PPER18784460
PMUM103328654
PAVI110764468
PDUL117621238
MDM103405762 103421889
PXB103936009 103948272
MNT21409733
CSV101218890
CMO103487508
BHJ120082736
MCHA111024979
CMAX111492838
CMOS111452318 111457768
CPEP111788701 111790346
RCU8265016
JCU105638783
HBR110659002
MESC110631370
POP7477453
PEU105133039
PALZ118055950
JRE108988141
QSU112028189
QLO115975759
TWL119995081 119998680
VVI100232933(OXC)
VRI117930698
SLY101264774
SPEN107011723
SOT102604551
CANN107860091
NTA107781829 107825460
NSY104211662
NTO104096832
NAU109243823
INI109154528
ITR116016327
SIND105176140
OEU111401032
EGT105961104
SSPL121746641 121750258 121779556 121783055
HAN110868206
ECAD122603179
LSV111903096
CCAV112501516
DCR108196317
CSIN114315157
BVG104906480
SOE110801650
CQI110703946 110720354
NNU104613342
MING122068615
TSS122645871 122650155
PSOM113282348 113341018 113355684
NCOL116248481
OSA4324491
DOSAOs01t0505400-01(Os01g0505400)
OBR102721129
BDI100829473
ATS109738743
TDC119270248 119278317
TAES123063247 123072164 123080437
SBI8056536
ZMA100191888
SITA101779082
SVS117857218
PVIR120672655 120705520
PHAI112895441
PDA103705628 103715163
EGU105047067 105047158
MUS103992223
DCT110114604 114579507
PEQ110035637
AOF109829098
ATR18428183
SMOSELMODRAFT_178690 SELMODRAFT_179619
PPP112279961
CSLCOCSUDRAFT_18867
APROF751_4156
GSLGasu_12440
SCEYEL020C(PXP1)
AGOAGOS_AEL055C
ERCEcym_1360
LTHKLTH0H14102g
VPOKpol_1061p50
ZROZYRO0E03454g
CGRCAGL0M03245g
NCSNCAS_0G03960(NCAS0G03960)
NDINDAI_0G00520(NDAI0G00520)
TPFTPHA_0B03940(TPHA0B03940)
TBLTBLA_0C01560(TBLA0C01560)
TDLTDEL_0B05520(TDEL0B05520)
TGBHG536_0G03620
KAFKAFR_0H02890(KAFR0H02890)
ZMKHG535_0B03790
PPAPAS_chr1-1_0394
YLIYALI0E07315g
SLBAWJ20_180
SSLSS1G_07774
BFUBCIN_15g01860
MBEMBM_05575
PSCOLY89DRAFT_234715
GLZGLAREA_03030
ANIAN1516.2
AFMAFUA_8G05230
ACTACLA_058670
NFINFIA_097990
AORAO090005000624
ANGANI_1_1960144(An16g07490)
AFVAFLA_003432
ALUCAKAW2_70591S
ACHEACHE_60592S
APUUAPUU_21693S
CIMCIMG_04305
CPWCPC735_074190
UREUREG_01556
PBLPAAG_11932(PAAG_04811)
PBNPADG_11446(PADG_02553)
ABEARB_06558
TVETRV_04424
AJEHCAG_02805
BGHBDBG_04203
ZTRMYCGRDRAFT_100226
PFJMYCFIDRAFT_158085
NPAUCRNP2_5114
TMLGSTUM_00003438001
SPOSPBC725.04
DDIDDB_G0292402
DPPDICPUDRAFT_156484
DFADFA_00674
ACANACA1_369090
TETTTHERM_00503839
PTMGSPATT00010458001
METRBSY238_2193
TBHTbon_01510
 » show all
Reference
1  [PMID:10468558]
  Authors
Foulon V, Antonenkov VD, Croes K, Waelkens E, Mannaerts GP, Van Veldhoven PP, Casteels M
  Title
Purification, molecular cloning, and expression of 2-hydroxyphytanoyl-CoA lyase, a peroxisomal thiamine pyrophosphate-dependent enzyme that catalyzes the carbon-carbon bond cleavage during alpha-oxidation of 3-methyl-branched fatty acids.
  Journal
Proc Natl Acad Sci U S A 96:10039-44 (1999)
DOI:10.1073/pnas.96.18.10039
  Sequence
[hsa:26061]
Reference
2  [PMID:15644336]
  Authors
Foulon V, Sniekers M, Huysmans E, Asselberghs S, Mahieu V, Mannaerts GP, Van Veldhoven PP, Casteels M.
  Title
Breakdown of 2-hydroxylated straight chain fatty acids via peroxisomal 2-hydroxyphytanoyl-CoA lyase: a revised pathway for the alpha-oxidation of straight chain fatty acids.
  Journal
J Biol Chem 280:9802-12 (2005)
DOI:10.1074/jbc.M413362200
Reference
3  [PMID:17956236]
  Authors
Casteels M, Sniekers M, Fraccascia P, Mannaerts GP, Van Veldhoven PP
  Title
The role of 2-hydroxyacyl-CoA lyase, a thiamin pyrophosphate-dependent enzyme, in the peroxisomal metabolism of 3-methyl-branched fatty acids and 2-hydroxy straight-chain fatty acids.
  Journal
Biochem Soc Trans 35:876-80 (2007)
DOI:10.1042/BST0350876
Other DBs
ExplorEnz - The Enzyme Database: 4.1.2.63
IUBMB Enzyme Nomenclature: 4.1.2.63
ExPASy - ENZYME nomenclature database: 4.1.2.63
BRENDA, the Enzyme Database: 4.1.2.63

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